Common Acquired and Congenital Anomalies Signs and Symptoms

(INFANGY) • Visible facial defect (cleft lip)

• Visible defect on physical examination (cleft
Congenital Anomalies palate)
• Sucking difficulties
• Occur in about 2% - 3% of all live births (Steele,
o Due to inability to create suction (cleft
1997) lip)
• Increases to 6% by 5 years • Swallowing difficulties (more in cleft
o When more anomalies are diagnosed palate); feeding difficulty (most
• Interdisciplinary team approach – vital for common prob. presented by parents)
providing holistic care: • Abdominal distention
o Surgical treatment • Susceptibility to infection
o Rehabilitation and education of the child • Respiratory (aspiration pneumonia),
• Mouth (oral thrush),
o Psychosocial & financial assistance for
• Ear (otitis media)
the parents
• Diagnostic Tests
▪ Parental disappointment and • Sonogram while in-utero
disillusion add to the complexity • Physical assessment
of the nursing care needed for Treatment
these infants • Correction/repair through plastic surgery in
later life
Gastrointestinal System Anomalies o Cheiloplasty – repair of cleft lip
Cleft Lip and Palate ▪ Done in infancy: 2 – 3 mos (2
• Cong. facial malformation characterized by non- – 10 weeks, Pilliteri)
fusion of facial processes (cleft lip), and non- ▪ Often follows “RULE OF 10”
fusion of tissue and bone of the hard and soft • 10 weeks old
palate (cleft palate) happening during embryonic • 10 lbs in weight
life • 10 gms in hemoglobin
• Uranoplasty
Etiology o Repair of cleft palate
• Defective development of embryonic primary o Best done in toddlerhood before
palate occurring on 7th to 8th week of fetal life, speech training period:
and defective development of embryonic • 18 – 36 months
secondary palate (cleft palate) occurring about
the 7th to 12th weeks of fetal life Complications
• Incidence: • Speech problems/faulty speech/speech
• Cleft lip more frequent, males disturbance
• Cleft palate higher in females • Hearing problems/loss
• Body image problems
Risk /Predisposing factors • Dental problem: malposition of teeth
• Multiple genetic factors • Infection
o Cleft lip – familial tendency
• Environmental factors: Nursing Care
• Maternal infection (viral infections) • Provide preoperative care:
• Radiation exposure • Careful feeding: small, frequent, slow w/
• Alcohol ingestion infant in upright position; w/ frequent burping,
• Tx w/ meds like: corticosteroids, some using appropriate feeding tools:
tranquilizers, antiepileptics o Cleft lip: rubber-tipped medicine
dropper or syringe
 o Cleft palate: large-holed, soft nipple
or paper cup
• Specialty feeding devices
Preoperative Care:
• Prepare parents for surgical procedures:
o Arrange for a multidisciplinary
meeting to discuss the short- and
long-term plans of care
• Provide psychological support:
o Be present during initial mother-
newborn contact
o Don’t show discomfort in caring for
infant
o Verbalize positive traits of infant
o Allow expression of feelings and
concerns
o Show pictures of “before and after
surgery”
Provide Postoperative Care:
• Maintain patent airway
• Position properly for drainage:
• Cleft lip: side-lying, never prone
• Cleft palate: prone, never supine
• Suction gently and carefully: insert suction
catheter along non-operative side
• Prevent injury to suture line:
• Maintain Logan bar over suture line post
cheiloplasty
• Proper position and positional changes
• Apply bilateral elbow restraints
• Remove 02 hrs, one @ a time, under
supervision
• Provide arm exercises
• Prevent sucking - use rubber- tipped
syringe/dropper, paper
• Minimize crying
• Anticipate needs
• Soothing tactile stimulation
• Diversion
• Play activities - exc. Pacifiers,
• teethers
Logan Bar - apparatus used to protect surgical
incision
• Provide mouth care/wash q after feeding, w/
water to prevent infection
• Avoid rubbing motion; pat gently to dry
• No fork, spoon, and straw, no ice drop
• Liquid diet, except milk
• Could form curds – difficult to clean
• Prevent infection
• Antibiotics as ordered
• Meticulous mouth care, suture line
cleansing and proper drying
Imperforate Anus
• A congenital anorectal malformation in w/c the
rectum ends in a blind pouch connecting it to the
vagina (recto-vaginal fistula), or to the urethra
(rectourethral fistula)
• Incidence:
• 1 in 5000 live births
• More commonly in boys
Etiology
• Failure of upper bowel to meet w/ pouch from
perineum 􏰀abnormal positioning of the caudal
hindgut by the anorectal septum in the 7th - 8th
week of gestation
Signs and Symptoms
• Absence of anal opening on inspection
• Non-insertion of rectal thermometer (not
practiced anymore)
• Progressive abdominal distention
• Difficult defecation or inability to defecate
• Absence of meconium passage in 1st 24 hours
• Passage of meconium from inappropriate
opening (vagina or urethra)
• (-) “wink” reflex
Diagnostic Tests/Procedures
• Wangesteen-rice method/ x-ray
• Sonogram
• Infant held upside – down
• To allow swallowed air to rise to the end of the
blind pouch of the bowel
• Helpful also to estimate distance the intestine is
separated from perineum or extent of correction
necessary
Treatment: Surgery
• Anoplasty for simple type
• Full-through operation w/ or w/o temporary
colostomy
Nursing Care • Abdominal distention
• Early detection • Feeding difficulties
• Observe newborn for passage of meconium 1st • Abdominal pain: irritability, crying
• Infants:
24 hrs – confirms anal patency
• Chronic constipation – hallmark of megacolon
• Provide preoperative care
• Explosive diarrhea
• Observe NPO; pacifier to suck • Bile-stained vomitus
• Maintain NGT to decompress stomach • Abdominal distention
• Provide warmth • Failure to thrive/malnutrition
• Monitor VS • Older children
• Prepare parent for surgery and for temporary • Chronic constipation
• Ribbon-like stools (pellet-like)
colostomy, if necessary
• Abdominal distention
• Provide postoperative care: • Palpable fecal mass
• Prevent infection • Visible peristalsis
• Meticulous skin care; perirectal care, observing • Fecal odor of breath
strict aseptic techniques • Anemia
• Administer and maintain IVF • malnutrition
• Monitor rate of flow; strict I&O measurement;
daily weight Diagnosis
• History and physical exams
• Provide oral feedings
• Barium enema
• w/ colostomy, begin oral feedings slowly once
• Abdominal x-ray
stools have been passed; I&O; w/ pull-throughs
• Rectal biopsy: confirms megacolon
– oral feedings slowly once peristalsis begins

Postoperative Care: Treatment: Surgery

• Preop care
• Provide parental teaching on colostomy care:
• NPO; pacifier
• Empty pouch as needed; skin care: • Prepare/assist NGT insertion
• Clean peristomal area w/ mild soap and water, • Monitor I&O
dry thoroughly; apply clean pouch • Provide warmth
• Use skin barrier as ordered • Meet emotional needs thru soothing touch,
• Psychological support: pacifier, consistent care
• Low-residue, high-CHON and high-calorie diet, if
• Visual and tactile stimulation
appropriate (childhood)
• Support parents
• Parenteral nutrition as ordered
• Bowel cleansing:
Hirschsprung’s Disease (Congenital Aganglionic • Liquid diet
Megacolon) • Stool softeners as ordered
• A mechanical obstruction of the bowels due to • Digital removal of stools
the absence of autonomic parasympathetic • Daily isotonic saline enemas/colonic irrigation
nerve ganglion cells in the distal bowel resulting
Post-op Care
in inadequate bowel motility
• Monitor respiratory status: VS, I&O, electrolytes,
• Etiology: unknown
stools
• Incidence:
• Maintain hydration and nutrition: monitor for
• Higher in children w/ Down’s syndrome • Higher
abdominal distention
in males – 4:1 male-female ratio
• Keep incision site clean and dry
• Predisposing/precipitating factors: • Familial
• Assess colostomy fxning; colostomy care:
factor, Down syndrome
meticulous skin care
• Pain relief: analgesics PRN
S/Sx:
• Newborns: • Prevent complications:
• No meconium stools • Respiratory infection: coughing, deep breathing,
• Bile-stained vomitus turning Q2H
 • Skin infection: meticulous skin care • Patency of • Observe for passage of stools or barium after
NGT hydrostatic reduction
• Psychological support • Postoperative care:
• Fluids and electrolytes status:
Intussusception • Urine output evidence of good hydration:
• Characterized by the invagination or telescoping 1mL/kg/hr
of the intestine along any point of the intestinal
tract (usually in the ileocecal valve), resulting in • Monitor return of bowel sounds and stools
intestinal obstruction and interference w/ the • Observe for sign of recurrent obstruction
passage of the intestinal contents
• Most common cause of bowel obstruction in Otitis Media
children • Inflammation of the middle ear
• Etiology:
• Most prevalent dse of childhood after RTI
• Generally unknown
• Associated w/ celiac disease, cystic fibrosis, and • (Kelley, 2008)
intestinal polyps • Extremely serious dse of childhood
• Permanent damage can occur to middle ear
Incidence: structures hearing impairment
• Hyperactivity of infant’s digestive tract ETIOLOGY:
intussusception
• Age of onset: 3 to 12 months • More common in • S. pneumoniae, H. influenzae (< 5 yrs old)
males (3:1) Incidence:
• 6 – 36 mos.; 4 – 6 yrs
S/Sx • Males; w/ cleft palate
• Recurrent colic • Formula-fed; constant pacifier use
• Abrupt intestinal pain elicited by pulling
knees up to the chest, as evidenced by • Held in a more slanted position – allows milk
crying and screaming • to enter the eustachian tube
• “Currant jelly” stools • Highest in winter and spring
• w/ blood, mucus, and pus • Homes in which a parent smokes cigarettes
• Abdominal distention w palpable “sausage
shaped” mass @ the right upper quadrant
Assessment
• Empty lower right quadrant = Dance sign
• Vomiting, prostration, schock • Usually occurs after a RTI (acute)
• Fever and progressive acute illness • “cold”, rhinitis and perhaps low grade fever x
several days
• Complications: • Temp: 38 ̊C and sharp, constant pain in 1 or
• Peritonitis, shock, or death if untreated both ears
• Dxnosis
• External ear – free of wax
• HX taking, clinical signs
• Barium enema • Tympanic membrane – inflamed or reddened
• May also reduce the intussusceptions • on otoscopic exam; bulging into ext. canal
• Tympanocentesis – obtain fluid for C&S
Treatment • Infants: extremely irritable;
• Barium or saline enema to effect hydrostatic • pull/tug @ affected ear
reduction of intussusceptions • To gain relief from pain
• Reduction by air pressure insufflations • Surgical
correction Therapeutic Management:
• If medical mgt is unsuccessful and child has • Most resolve spontaneously w/o therapy
peritonitis and shock • Ampicillin, Amoxicillin (for H. influenzae)
• Today – antibiotic therapy may add to
Nursing Care bacterial resistance = no longer routinely
prescribed
• Monitor VS, electrolyte levels, urine, stools
• Analgesic, antipyretic
• Psychological support
 • Nasal decongestant only for 3 days (rebound
effect)
Otovent (For Otitis Media)
• Otovent can prevent or treat Otitis media and
can be an attractive alternative to surgery.
• The new Otovent method is designed to treat
many of the complications associated with
negative ear pressure caused by Eustachian
tube dysfunction.
Therapeutic Management
• Parents’ education:
o Caution parents about conductive
hearing loss up to 6 mos. after acute
infection
o Possible hearing loss
o Conductive hearing loss after 6 mos
(or has other sx) = shd be examined
again for new infection or serrous
otitis media
• Chronic or persistent otitis media =
staphylococcus
o Tx: Cephalosporin
Otitis Media w/ Effusion (OME)
• occurs when fluid, called an effusion,
becomes trapped behind the eardrum in one
or both ears, even when there is no infection.
• In chronic and severe cases, the fluid is very
sticky and is commonly called "glue ear.”
Pathophysiology
Middle ear-air filled cavity w/eustachian tube
supplying the air — Tube opens and closes
w/yawning, swallowing, or chewing — Source of air
shut off (chronic otitis media, allergy, inflammation)
— Epithelial cells of middle ear change fxn= become
secretory — Middle ear fills w/secretions — Fluid
becomes so thick and tenacious = “gluelike
” • small tubes, open at both ends, that are
Signs and Symptoms
• usually not painful
• child complains of "plugged up" hearing
• Feeling like "being under water."
• may impair children's hearing
o loud talking, not responding to verbal
commands, and turning up the
television or radio.
• Most episodes resolve w/in 3 months
o 30 - 40% of children may have
recurrent episodes.
o Only 5 - 10% of episodes last longer
than 1 year.
Other S/Sx
• Sound of popping or ringing in the ears
• Drop in hearing of 20 – 40 dB
• Due to fluid content
• Occurs most frequently in children 3 -10 yrs
of age
• Purulent middle ear effusion and tympanic
membrane with a loss of landmarks and
characteristic bagel or doughnut appearance
• Acute otitis media with effusion. There is
distortion of the drum, prominent blood
vessels in the upper half, with dullness of the
lower half. There is also bulging of the upper
half of the drum and the outline of the
malleus is obscured.
Therapeutic Management
• Aimed @ supplying air to the middle ear
• Mild involvement:
o Daily antihistamine or nasal
decongestant
o shrink mucous membrane of
eustachian tube
• Enlarged adenoids: adenoidectomy
• Fluid from middle ear:
o Tympanocentesis
• Tubal Myringotomy
• surgical procedure in which a small incision
is made in the eardrum (the tympanic
membrane), usually in both ears.
• comes from myringa, modern Latin for drum
membrane, and tome, Greek for cutting.
• also called myringocentesis, tympanotomy,
tympanostomy, or paracentesis of the
tympanic membrane.
• Fluid in the middle ear can be sucked out
through the incision.
• Ear tubes, or tympanostomy tubes
inserted into the incisions in the eardrums
during myringotomy.
• come in various shapes and sizes and are
made of plastic, metal, or both.
• left in place until they fall out by themselves
or until they are removed by a doctor.
• When myringotomy tubes are in place:
• Water shd not be allowed to enter child’s ears
• Bathe rather than shower (allowed if w/ ear
plugs)
• Swimming and hair washing – w/ ear plugs
• Teach parent to continue meds
• support to accept insertion of
myringotomy tubes, and other
procedures (cutting of eardrum)